Description

• One of the causes of critical limb ischemia
• Nonatherosclerotic vasculitis of small- and medium-sized arteries and veins resulting in segmental occlusion of the distal extremity vasculature; caused by inflammatory thrombo-occlusive disease
• Characterized by resting pain, ischemic ulcerations, and gangrene of the digits of hands and feet
• Patients are typically young (age <50 years), male smokers.
• Synonym(s): Buerger disease

Epidemiology

• The prevalence has decreased in North America over the past 30 years.
• Most prevalent in Eastern Europe, Mediterranean, and Asian countries

Incidence

• 11 to 30/100,000 persons per year
• Predominant age: 20 to 40 years
• Predominant sex: male > female; increasingly diagnosed in women, perhaps due to increased smoking

Prevalence

• Estimates range from as low as 0.5–5.5% in Western Europe, to 45–63% in India, to 80% in Israel among those of Ashkenazi ancestry.
• Accounts for 5% and 16% of patients hospitalized for arterial occlusive disease in Europe and Japan, respectively
• 13/100,000 U.S. population
• Overall occurrence is decreasing worldwide.

Geriatric Considerations
Not common in the elderly

Pediatric Considerations
Consider in adolescent smokers presenting with claudication, digital ulcers, or digital gangrene; rare in children

Etiology and Pathophysiology

• Thromboangiitis obliterans (TAO) is idiopathic in nature. Demonstrates impaired endothelium-dependent vasorelaxation and decreased peripheral sympathetic outflow. Nonendothelial mechanisms of vasodilation are intact.
• Segmental infiltration of inflammatory cells in vessel wall leads to thrombotic occlusion.
• Highly cellular and inflammatory thrombus with relative sparing of the blood vessel wall
• Smoking predisposes to occurrence.
• Genetic factors implicated
• Autoimmune component which is not fully understood
• Chronic anaerobic periodontal infection

Genetics

• Greater prevalence of HLA-A54, HLA-A9, and HLA-B5
• HLA-B12 antigen may be associated resistance to disease.
• Familial cases rarely reported

Risk Factors

• Smoking as little as 1 to 2 cigarettes daily, chewing tobacco, snuff, and nicotine replacement are all risk factors for TAO.
• Chronic anaerobic periodontal infection also may play a role in the development of TAO.

General Prevention

Tobacco cessation